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30 Cards in this Set

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At what age do T-cell immunodeficiencies present and what types of infections are they characterized by?
1-3 months, broad range infections (fungal, bacterial, viral)
List two of the most common T-cell immunodeficiency syndromes
DiGeorge's syndrome and ataxia telangiectasia
Waht is the embryonal deformity in DiGeorge's syndrome?
Agenesis of the third and fourth pharyngeal pouch (responsible for the development of the thymus and parathyroid gland)
What are the clinical manifestations of DiGeorge syndrome?
"CATCH -22"
Carotid anomalies (tetralogy of Fallot, interrupted aortic arch, and vascular rings)
Abnormal facies
Thymic hypoplasia
Cleft palate
Hypocalcemia
22 (chromosome 22q11 microdeletion)
Name the immunodeficiency syndrome characterized by cerebellar ataxia, oculocutaneous telangiectasia, decreased T-cell function, and low antibody levels.
Ataxia telangiectasia
At what age do B-cell deficiency syndromes typically present?
6 months (maternal antibodies protect infant up to this age)
What types of infection occur in B-cell deficiency syndromes?
Recurrent upper respiratory infections and bacteremia caused by encapuslated organisms
Name the three most common B-cell deficiency syndromes
X-linked aggamaglobulinemia, common-variable immunodeficiency, and selective IgA deficiency
Name the disease characterized by a total lack of antibody production
X-linked (Bruton's) aggamaglobulinemia
Name the disease characterized by recurrent respiratory, GI, and urinary tract infections
Selective IgA deficiency
List the two most common combined B- and T- cell immunodeficiency syndromes
Severe combined immunodeficiency disease (SCID) and Wiskott-Aldrich syndrome
What are the clinical manifestations of Wiskott-Aldrich syndrome?
"WATER"
W - decreased IgM
A - increased IgA
Thrombocytopenia
Eczema
Recurrent infections
What are the clinical manifestations of phagocytic immunodeficiency syndromes?
Poor wound healing, abscess formation, and granulomas
Name the two most common phagocytic syndromes.
Chronic granulomatous disease (X-linked recessive) and Chediak-Higashi syndrome (AR)
What chemial process is affected in patients with chronic granulomatous?
Oxidative burst that produces hydrogen peroxide
What is the defective immunologic process of Chediak - Higashi syndrome?
Neutrophil chemotaxis (may manifest as occulocutaneous albinism)
What pediatric disease is characterized by multijoint pain, fatigue, rash, lymphadenopathy, and failure to thrive?
Juvenile rheumatoid arthritis (JRA)
Name the type of JRA associated with each of the following statements:
5+ joints, symmetric; resembles adult RA; +/- rheumatoid factor (RF)
Polyarticular
Name the type of JRA associated with each of the following statements:
Small, pink, salmon-colored macular rash; high-spiking fevers, hepatospelenomegaly (HSM)
Systemic (10-20%), "Still's disease"
Name the type of JRA associated with each of the following statements:
<5 joints (usually weight-bearing); type 1=females <4 y/o; type 2 =males > 8 y/o
Pauciarticular (~50%)
What is the treatment for JRA?
NSAIDs and strengthening exercises; methotrexate (MTX) = second line
What are the major complications of systemic JRA?
Pulmonary, hepatic, CNS, Nerve entrapment (eg carpal tunnel syndrome)
What lab value is significantly increased in patients with dermatomyositis?
Serum creatinine kinase
Name the vasculitis characterized by each of the following:
Positive cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA)
Wegener's granulomatosis
Name the vasculitis characterized by each of the following:
Palpable purpura, abdominal pain, and hematuria
Henoch-Schonlein purpura
Name the vasculitis characterized by each of the following:
Recurrent upper and lower respiratory tract infections
Wegener's granulomatosis
Name the vasculitis characterized by each of the following:
Treated with IV immunoglobulin (IVIG) and high-dose aspirin
Kawasaki disease
Name the vasculitis characterized by each of the following:
Treated with corticosteroids and cyclophosphamide
Wegener's granulomatosis
Name the vasculitis characterized by each of the following:
Coronary aneurysms are the most fatal complicaiton
Kawasaki disease
What are the clinical manifestations of Kawasaki disease?
"My HEART"
My- Mucous membrane changes (fissured lips and strawberry tongue)
Hands and extremity changes (erythema and edema)
Eye changes (conjunctivitis with limbal sparing)
Adenitis (painful lymphadenitis)
Rash
Temperature (>104F for 5 days)